What causes pulmonary hypertension?

Pulmonary hypertension (PH) is a complex and often misunderstood disease. PH means high blood pressure in the lungs. PH is different from regular hypertension. In regular hypertension (also known as high blood pressure), the arteries throughout a person’s body are constricted. In pulmonary hypertension, the blood vessels in the lungs become narrowed and the heart has to work harder to pump blood through them.

In many cases, no cause can be identified, and in these cases the disease is referred to as idiopathic pulmonary arterial hypertension (IPAH). Sometimes PH can develop due to another medical condition, including connective tissue disease, congenital heart disease, liver disease, HIV, and others. This type of PH is known as associated pulmonary arterial hypertension (APAH). Some families have a form of PH that can be inherited. This is known as heritable pulmonary arterial hypertension (HPAH). Chronic thromboembolic pulmonary hypertension (CTEPH) is a form of pulmonary hypertension caused by old blood clots in the lungs (pulmonary embolism).

How does my doctor know I have PH?

PH can be difficult to diagnose in a routine medical exam because the most common symptoms of PH are also associated with many other conditions. To determine if you have PH, your medical team will schedule specialized tests, possibly to include a pulmonary function test and an echocardiogram. If the results of these tests point to PH, your doctor will schedule a cardiac catheterization, sometimes referred to as a “right-heart cath.” This test allows your doctor to directly measure the pressures in your heart and lungs. Right heart catheterization is the gold standard for diagnosing PH.

Could PH run in my family?

Yes, but heritable PAH (HPAH) is relatively uncommon. Of the small percentage of people who do carry the PH gene, only a small number of carriers will develop the disease. Genetic testing is available to find out if you carry the PH gene.

What kinds of doctors treat PH?

PH is a rare but serious disease, and many doctors, including cardiologists and pulmonologists, have little training or experience in diagnosing and treating PH. Patients are encouraged to see a cardiologist, pulmonologist or, in some cases, a rheumatologist who treats many PH patients. These doctors are familiar with PH medications and side-effects, and can determine which treatments or combination of therapies are right for you.

How can I find a doctor to treat my PH?

One way to begin your search for a PH-treating doctor is through PHA's Find a Doctor directory. Once you’ve found a list of physicians in your area, interview them carefully to determine which center will offer you the most specialized care.

Here are some questions to pose to doctors you’re considering:

Are you part of a dedicated PH clinic that includes other PH-treating doctors?

Do you have at least one nurse who works with patients on PH-specific medications?

How many PH patients do you currently treat?

What PH therapies do you prescribe, and how many patients are on IV prostacyclin? (While you may ultimately be placed on a different therapy, a center’s familiarity with treating patients on the most complex PH therapy is a good reflection of their level of PH expertise.)

Does your center perform right heart catheterization with vasodilator testing, and who does the catheterizations?

Do you conduct clinical trials on PH medications?

What questions should I be asking my medical team?

Keep a list of your questions between appointments, and don’t be afraid to ask why your doctor is running a test, scheduling a procedure, or changing a medication. At your first appointment, you might consider asking: How often should I come to see you? How often do I need to have cardiac catheterization? What are the potential side-effects of my medication? If you wear a pump for your medication, you might want to ask: What do I do if the line leaks or comes out? How do I need to alter my daily routine to accommodate the pump? You may also want to discuss lifestyle changes, such as nutrition and exercise, with your PH team.

What medications are available to treat PH?

A number of FDA-approved medications are available for the treatment of PH. These medications are administered in several ways: directly into the vein (intravenously), beneath the skin (subcutaneously), orally, and by inhalation.

Some PH medications work by mimicking prostaglandin, a substance that PH patients tend to be deficient in. These treatments are called are prostacyclins, and include epoprostenol (Flolan™), treprostinil (Remodulin™ and Tyvaso™), and iloprost (Ventavis™).

Other PH medications work by reversing the effects of endothelin, a substance that PH patients tend to have in excess. These are called endothelin receptor antagonists, and include bosentan (Tracleer™) and ambrisentan (Letairis®).

Still other PH treatments work by allowing the lungs to produce more of their own natural vasodilators. Called PDE 5 Inhibitors, this category of treatment includes sildenafil (Revatio™) and tadalafil (Adcirca™).

Doctors may also prescribe the anticoagulant warfarin (Coumadin™) to prevent blood clots, diuretics to reduce fluid retention, and supplemental oxygen to help patients breathe.

How will my doctor determine which treatment is best for me?

Your doctor will take into consideration the severity of your illness (referred to as your “functional class”) and the results of your cardiac catheterization to help determine which medication is right for you. As your symptoms and pressures change, your doctor may want to adjust the type and dosage of your medication accordingly.

What's the outlook for someone with PH?

There’s no simple answer to this question. Every PH patient is different, and new research with the potential to improve the outlook for this disease is being conducted all the time. Your journey with PH depends on many factors, including the severity of your disease and how you respond to treatment. PH-specific therapies are available that can prolong and improve your quality of life. Once in the care of a PH specialist and on treatment, many PH patients live for many years.

How will PH affect my life?

While it can be difficult to imagine in the days and months following diagnosis, many patients develop strategies to cope with the physical and emotional aspects of living with PH and lead happy, fulfilling lives. Making small changes in daily activities can make a big impact on how you feel. Listen to your body and rest when you need to. Talk to your doctor about the possibility of supplemental oxygen if you haven’t already. With a bit of patience, planning, and flexibility, many patients find ways to work, travel, exercise, and do the things they loved before diagnosis. Visit the Living with PH section for more tips on living with PH.

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The information provided on the PHA website is provided for general information only. It is not intended as legal, medical or other professional advice, and should not be relied upon as a substitute for consultations with qualified professionals who are familiar with your individual needs. PHA does not endorse or recommend any commercial products or services.